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ABSTRACT Objective: To describe the clinical features and outcomes of patients with uveitis associated with juvenile idiopathic arthritis (JIA) and idiopathic uveitis. Methods: This was an observational, retrospective study, conducted in a tertiary center. Patients under 18 years old who experienced at least one episode of uveitis and followed between 2000 and 2019 were included. Results: A total of 82 patients were included, of whom 43 had idiopathic uveitis and 39 had uveitis associated with JIA. Anterior uveitis was the primary site of ocular inflammation (76.8%) and occurred in 24 and 39 patients with idiopathic uveitis and uveitis associated with JIA arthritis, respectively (p=0.02). The complete response to corticotherapy was more frequent in uveitis associated with JIA (p=0.001). Total and partial responses to biological disease modifying antirheumatic drugs were more frequent in uveitis associated with JIA (p=0.025) and idiopathic uveitis (p=0.045), respectively. There were 203 complications: cataracts were more frequently present in idiopathic uveitis (p=0.05), while synechiae was more frequent in uveitis associated with JIA (p=0.02). Conclusion: Idiopathic uveitis and uveitis associated JIA frequently follow a chronic course and an increased risk of visual loss in childhood. The uveitis associated with JIA showed better response to systemic corticotherapy and total response to biologic disease modifying antirheumatic drugs more frequently.
RESUMO Objetivos: Descrever as características clínicas e desfechos dos pacientes com uveíte associada à Artrite Idiopática Juvenil (AIJ) e da Uveíte Idiopática. Métodos: Este foi um estudo retrospectivo observacional conduzido em um centro terciário. Foram incluídos pacientes abaixo dos 18 anos de idade que apresentaram pelo menos um episódio de uveíte e que estiveram em acompanhamento médico entre os anos de 2000 e 2019. Resultados: Foram incluídos 82 pacientes, sendo 43 com uveíte idiopática e 39 com uveíte associada à AIJ. A uveíte anterior foi o sítio primário de acometimento (76,8%) em 24 e 39 pacientes com uveíte idiopática e uveíte associada à AIJ, respectivamente (p=0.02). Resposta total à corticoterapia foi mais frequente na uveíte associada à AIJ (p=0.001). Respostas total e parcial às drogas antirreumáticas modificadoras de doença biológicas foram mais frequentes na uveíte associada à AIJ (p=0.025) e na uveíte idiopática (p=0.045), respectivamente. Foram encontradas 203 complicações: a catarata foi mais frequente na uveíte idiopática (p=0.05), enquanto a sinéquia foi mais frequente na uveíte associada à AIJ (p=0.02). Conclusão: A uveíte idiopática e a uveíte associada à AIJ frequentemente apresentam um curso crônico e um risco elevado de perda visual na infância. A uveíte associada à AIJ apresentou melhor resposta à corticoterapia sistêmica e resposta total às drogas modificadoras de doença reumática biológicas mais frequentemente.
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Objective:To explore the differences of clinical laboratory indicators between Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (SJIA), providing objective evidence for diagnosis and differential diagnosis of these diseases.Methods:A total of 41 children patients with KD (KD group) and 33 children patients with SJIA (SJIA group) who received treatment in Huainan Maternal and Child Health Hospital between September 2017 and January 2022 were retrospectively analyzed. An additional 50 healthy children who concurrently received physical examination in the same hospital were included in the control group. Platelet count (PLT), white blood cell count (WBC), and erythrocyte sedimentation rate (ESR) as well as C-reactive protein (CRP), serum procalcitonin (PCT), interleukin-6 (IL-6), interleukin-10 (IL-10), and serum ferritin (SF) levels were compared among groups before treatment.Results:One-way analysis of variance and pairwise q test were performed to compare laboratory indicators among KD, SJIA and control groups. CRP, ESR, SF and IL-6 levels in the KD group were significantly lower than those in the SJIA group [CRP: (57.80 ± 25.23) mg/L vs. (77.72 ± 45.64) mg/L; ESR: (67.02 ± 28.80) mm/h vs. (83.84 ± 47.64) mm/h; SF: (320.21 ± 182.53) μg/L vs. (945.58 ± 604.65) μg/L; IL-6: (50.35 ± 20.54) ng/L vs. (89.35 ± 45.54) ng/L, q = 4.34, 3.42, 11.51, 8.85, all P < 0.05]. IL-10 level in the KD group was significantly higher than that in the SJIA group [(18.52 ± 16.71) ng/L vs. (10.01 ± 3.24) ng/L, q = -5.25, P < 0.05]. WBC, CRP, ESR, PCT, PLT, IL-6, IL-10 and SF in the KD and SJIA groups were significantly higher than those in the control group (all P < 0.05). Conclusion:Detection of CRP, ESR, SF, IL-6, IL-10 in blood can provide objective evidence for the early diagnosis and differential diagnosis of KD and SJIA, thereby reducing the misjudgment of clinical diagnosis.
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Juvenile idiopathic arthritis (JIA) is a genetically heterogeneous group of connective tissue diseases that are commonly characterized by chronic joint synovial inflammation with unknown etiology in childhood.It is cu-rrently incurable and the main therapeutic goal is to achieve clinical remission.The drugs currently used to treat JIA mainly include non-steroid anti-inflammatory drugs, glucocorticoid, disease modifying antirheumatic drugs, and biological agents.In this article, recent advances in the understanding of JIA treatment and related clinical research were reviewed, in an attempt to provide prospects for the future direction of drug development and treatment concepts.
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Objective:To analyze cytokine pattern of systemic onset juvenile idiopathic arthritis (SOJIA) combined with macrophage activation syndrome (MAS) in children, and study the early diagnostic value in MAS.Methods:The clinical data of 157 children with SOJIA from January 2013 to March 2018 in Taizhou Hospital of Zhejiang Province were retrospectively analyzed. Among them, SOJIA combined with MAS was in 15 cases (SOJIA combined with MAS group), and simple SOJIA was in 142 cases (simple SOJIA group). The peripheral blood levels of interleukin (IL)-2, IL-4, IL-6, tumor necrosis factor (TNF)-α and interferon (IFN)-γ were measured by flow cytometry cytometric beads array. The characteristics of cytokine pattern was analyzed.Results:The IL-10 and IFN-γ in SOJIA combined with MAS group were significantly higher than those in simple SOJIA group: 40.5 (7.9, 236.9) ng/L vs. 4.1 (2.0, 98.7) ng/L and 55.8 (18.5, 500.0) ng/L vs. 4.4 (1.4, 30.1) ng/L, and there were statistical differences ( P<0.01); there were no statistical difference in IL-2, IL-4, IL-6 and TNF-α between 2 groups (P>0.05). Pearson correlation analysis result showed that IL-10 was positive correlated with IFN-γ in SOJIA children with MAS ( r = 0.638, P = 0.011). Receiver operating characteristic curve analysis result showed that the area under the curve (AUC) of IFN-γ in predicting MAS was 0.991, 95% CI 0.974 to 1.000, the optimal critical value was 18.45 ng/L, the sensitivity was 92.5%, and the specificity was 95.1%; the AUC of IL-10 in predicting MAS was 0.944 (95% CI 0.893 to 0.996), the optimal critical value was 7.75 ng/L, the sensitivity was 91.7%, and the specificity was 81.7%. Conclusions:The significant increased IL-10 and IFN-γ is helpful for the early diagnosis MAS in children with SOJIA.
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Objective:To investigate the clinical characters and prognosis of patients with systemic-onset juvenile idiopathic arthritis associated interstitial lung disease (SoJIA-ILD).Methods:Clinical manifestations, imaging features and prognosis were analyzed in 75 patients with SoJIA between October 2010 and December 2019 in the Second Affiliated Hospital of Wenzhou Medical University.Results:Seventy-five patients with SoJIA were enrolled. Among 12 children with SoJIA-ILD, 6 were male and 6 were female. The mean age of onset was (7.9±2.6) years. All of the 12 patients had fever. Ten patients had arthritis which mainly occurred in large joints. The incidence of arthritis was knee, hip and shoulder from high to low. Two patients had no joint involvement at the onset of the disease and had no joint symptoms during the follow-up. Nine patients (75%) had fever, rash and arthritis at the same time. The clinical features of ILD were mostly nonspecific, including cough in 8 cases (75%), shortness of breath in 7 cases (58%), chest pain in 3 cases (25%), velcro sound in 4 cases (33%) and pulmonary hypertension in 1 case (8%). Inflammatory indicators were all signifi-cantly elevated, among which was CRP (235±112) mg/L, ESR (39±25) mm/1 h, serum ferritin (SF) (1 312±384) ng/ml and serum amyloid A (SAA) (212±101) mg/L. High resolution computed tomography (HRCT) of the chest presented as reticular or line shadows in 12 patients, consolidation in 7 patients, ground interlobular septal thickening in 5 patients, glass opacity in 4 patients and honeycomb lung in 1 patient. ILD occurred in 4 cases (33%) in the early stage of SoJIA (disease course ≤6 months), and 8 cases (67%) in the medium and late stages of the disease course (>6 months), but all appeared in the active status of SoJIA. All of 12 patients received glucocorticoids therapy, 11 patients received high dose of glucocorticoid (>1 mg·kg -1·d -1) and 2 pa-tients received intravenous methylprednisolone pulse therapy. All of 12 patients were treated with glucocorti-coids combined with immunosuppressant or disease modifying antirheumatic drugs and 5 patients needed dual therapy or triple therapy. One case had been treated with biological agents before the occurrence of lung injury and the other 11 cases had not used biological agents before. After the diagnosis of SoJIA complicated with ILD, 4 cases were treated with tocilizumab. Macrophage activation syndrome (MAS) was found in 7 cases and 25% had MAS for two times or more. Ten patients had partial remission or complete remission and 2 patients died of respiratory failure. Conclusion:SoJIA-ILD maybe asymptomatic at the early stage of the disease. It is associated with disease activity of SoJIA. HRCT examination is very important for early diagnosis. Patients with SoJIA-ILD have higher rate of recurrence, death and MAS. It needs to arouse the clinicians' attention.
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Abstract Hip pain in a child can have infectious, inflammatory, traumatic, neoplastic, or developmental causes, which can make the diagnosis challenging. Meticulous history taking and a detailed clinical examination guide the radiological investigation. In this article, we address some of the main causes of hip pain in childhood and their findings on diagnostic imaging.
Resumo A dor do quadril na criança pode resultar de causas infecciosas, inflamatórias, traumáticas, neoplásicas ou de desenvolvimento, por vezes gerando desafios diagnósticos. Uma história meticulosa e um exame clínico detalhado orientam a investigação radiológica na direção apropriada. Neste artigo abordaremos algumas das principais doenças do quadril doloroso na criança e seus achados nos exames de imagem.
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Objective By studying the efficacy of interleukin (IL)-6 receptor antagonist (tocilizumab) on acute inflammation of systemin juvenile id-iopathic arthritis (sJIA) and its effect on the downstream signaling pathways and inflammatory factors of IL-6 to further reveal the role of tocilizumab in sJIA.Methods From December 2015 to December 2018,64 sJIA children were randomly divided into two groups:31 cases who were treated with tocilizumab+ glucocorticoid+disease-modifying anti-rheumatic drugs (DMARDs) as the tocilizumab group,33 cases who were treated with placebo (vitamin C) + glucocorticoid+DMARDs as the control group.They were treated for one year.The levels of IL-2,IL-4,IL-6,IL-10 and tumor necrosis factor (TNF)-α were detected by enzyme-linked immunosorbent assay (ELISA).The expressions of p65 and receptor activator for nuclear factor-κB ligand (RANKL) in peripheral blood mononuclear cells (PBMCs) were detected by quantitative polymerase chain reaction (qPCR).The expressions of signal transducer and activator of transcription (STAT3)/phosphates signal transducer and activator of transcription 3 (p-STAT3)/suppressor of cytokine signaling 3 (SOCS3) before and after treatment were detected by Western blotting.The differences between groups were analyzed by variance analysis.Normal distributed data was tested by K-W test.Twenty normal control subjects came from the pediatric clinic in our hospital.Results There was no significant difference in the demographic data between the two groups (P＞0.05).Among them,2 children who were treated with tocilizumab dropped out after one month treatment and three months due to un-affordability respectively.The C-reactive protein (CRP),ferritin (FER),erythrocyte sedimentation rate (ESR) in the tocilizumab treatment group decreased significantly after 6 months and 1 year when compared with the disease control group.The concentration of IL-6 in the tocilizumab group (77±46) pg/ml,control group (82±40) pg/ml were higher than that in the healthy control group (10±3) pg/ml (F=4.683,P=0.001;F=2.581,P=0.03).After one year,the concentration of IL-6 (316±42) pg/ml in the tocilizumab group was higher than that in the disease control group (62±40) pg/ml (F=11.2,P=0.001).The expression of RANKL and p65 mRNA in treatment group was significantly higher than that in healthy control group (K-W=10.03,P＜0.01;K-W=9.42,P＜0.01).After one year,the expression of RANKL and p65 mRNA in treatment group was lower than that in disease control group (K-W=9.964,P＜0.01;K-W=10.75,P＜0.01).The expression of STAT3/p-STAT3/SOCS3 in disease control group before medication was significantly higher than that in healthy control group,while the expression of p-STAT3/SOCS3 in the treatment group was significantly higher than that in healthy control group.The expression of STAT3/p-STAT3 in the tocilizumab group was significantly lower than that in the disease control group (K-W=12.54,P＜0.01;K-W=10.52,P＜0.01).Conclusion Tocilizumab can effectively alleviate the symptoms of sJIA in active phase,down-regulate the expression of STAT3/p-STAT3 protein,thereby reducing the transcription of downstream nuclear factor (p65,RANKL) mRNA,thereby affecting the proliferation of synovial cells and reducing bone destruction,but has no significant effect on the secretion of IL-6.
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Objective@#To investigate the early typing diagnostic and predictive value of anti-keratin antibodies(AKA), anti-perinuclear factor(APF) and anti-citrullinated protein antibodies(ACPA) in patients of juvenile idiopathic arthritis (JIA).@*Methods@#A retrospective study was conducted to collect 144 cases of JIA who were hospitalized in Capital Institute of Pediatrics from December 2013 to June 2016 and followed up for at least one year.Among them,66 were males (46%) and 78 were females (54%).The age at diagnosis was between 1 year 5 months to 15 years 9 months.144 patients were tested for AKA,ACPA,APF and TNFα upon admission. Chi-square test or Fisher exact test were used to compare the positive rates of three antibodies among different subtypes. Mann-Whitney nonparametric test and Chi-square test or Fisher exact test were used to analyze the data of prognosis between antibody-positive group and antibody-negative group in the course of disease.@*Results@#In 144 patients, 49(34%) were classified as systemic arthritis, 28 (19.4%) as polyarthritis, 61(42.3%) as oligoarthritis, and 6(4.2%) as enthesitis-associated arthritis. 52 cases (36.1%) were positive for one antibody or more antibodies of AKA/APF/ACPA at the early stage, 14(9.7%) were AKA positive, 44(30.6%) were ACPA positive and 12(8.3%) were APF positive. The positive rates of ACPA/AKA/APF antibodies were significantly different among different subtypes(χ2=33.863,26.860,14.395; P<0.01,<0.01,<0.05).The rates in polyarthritis were higher than those in systemic arthritis and oligoarthritis; In 95 children with non-systemic form, the level of TNFα in antibody-positive group (43 cases) was higher than that in antibody-negative group (52 cases) at the early stage(Z=4.785, P<0.01);144 patients were followed up for at least one year,the rates of patients who accepted biologic therapies were significantly different between antibody-positive group and antibody-negative group (50% vs 25%). So do the rates of patients with joint deformities (17.3% vs 2.2%) and with important joints involvement (hip and axis joints) (59.6% vs 14.1%) (χ2=9.249,10.875,32.392; P<0.01,<0.01,<0.01). Further more, the number of joints involved in the antibody-positive group (7.07±3.85) was significantly more than that in the antibody-negative group (2.31±1.64) (F=63.822, P<0.01).@*Conclusions@#AKA,APF and ACPA are important in the early typing diagnosis of JIA,and may be closely related to the prognosis of patients with JIA.
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Objective@#By studying the efficacy of interleukin (IL)-6 receptor antagonist (tocilizumab) on acute inflammation of systemin juvenile id-iopathic arthritis (sJIA) and its effect on the downstream signaling pathways and inflammatory factors of IL-6 to further reveal the role of tocilizumab in sJIA.@*Methods@#From December 2015 to December 2018, 64 sJIA children were randomly divided into two groups: 31 cases who were treated with tocilizumab+ glucocorticoid+disease-modifying anti-rheumatic drugs (DMARDs) as the tocilizumab group, 33 cases who were treated with placebo (vitamin C) + glucocorticoid+DMARDs as the control group. They were treated for one year. The levels of IL-2, IL-4, IL-6, IL-10 and tumor necrosis factor (TNF)-α were detected by enzyme-linked immunosorbent assay (ELISA). The expressions of p65 and receptor activator for nuclear factor-κB ligand (RANKL) in peripheral blood mononuclear cells (PBMCs) were detected by quantitative polymerase chain reaction (qPCR). The expressions of signal transducer and activator of transcription (STAT3)/phosphates signal transducer and activator of transcription 3 (p-STAT3)/suppressor of cytokine signaling 3 (SOCS3) before and after treatment were detected by Western blotting. The differences between groups were analyzed by variance analysis. Normal distributed data was tested by K-W test. Twenty normal control subjects came from the pediatric clinic in our hospital.@*Results@#There was no significant difference in the demographic data between the two groups (P>0.05). Among them, 2 children who were treated with tocilizumab dropped out after one month treatment and three months due to un-affordability respectively. The C-reactive protein (CRP), ferritin (FER), erythrocyte sedimentation rate (ESR) in the tocilizumab treatment group decreased significantly after 6 months and 1 year when compared with the disease control group. The concentration of IL-6 in the tocilizumab group (77±46) pg/ml, control group (82±40) pg/ml were higher than that in the healthy control group (10±3) pg/ml (F=4.683, P=0.001; F=2.581, P=0.03). After one year, the concentration of IL-6 (316±42) pg/ml in the tocilizumab group was higher than that in the disease control group (62±40) pg/ml (F=11.2, P=0.001). The expression of RANKL and p65 mRNA in treatment group was significantly higher than that in healthy control group (K-W=10.03, P<0.01; K-W=9.42, P<0.01). After one year, the expression of RANKL and p65 mRNA in treatment group was lower than that in disease control group (K-W=9.964, P<0.01; K-W=10.75, P<0.01). The expression of STAT3/p-STAT3/SOCS3 in disease control group before medication was significantly higher than that in healthy control group, while the expression of p-STAT3/SOCS3 in the treatment group was significantly higher than that in healthy control group. The expression of STAT3/p-STAT3 in the tocilizumab group was significantly lower than that in the disease control group (K-W=12.54, P<0.01; K-W=10.52, P<0.01).@*Conclusion@#Tocilizumab can effectively alleviate the symptoms of sJIA in active phase, down-regulate the expression of STAT3/p-STAT3 protein, thereby reducing the transcription of downstream nuclear factor (p65, RANKL) mRNA, thereby affecting the proliferation of synovial cells and reducing bone destruction, but has no significant effect on the secretion of IL-6.
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A nine-year-old girl with of juvenile idiopathic arthritis (JIA) in use of methotrexate complained of a self-bleeding ulcerated lesion exhibiting a red surface in the lower lip. Pyogenic granuloma was confirmed by histopathological analysis. After the 7th and 15th days, the lip where biopsy had been done exhibited a deficient healing. The case was discussed with the physician for temporary suspension of methotrexate. Complete wound healing of the biopsied site occurred after temporary interruption of the medication. Wound healing after methotrexate temporary suspension allowed concluding that the drug delayed surgical wound healing was a consequence of drug cytotoxicity. (AU)
Uma menina de nove anos de idade com artrite idiopática juvenil (AIJ) em uso de metotrexato queixou-se de lesão ulcerada com sangramento espontâneo que exibia superfície avermelhada em lábio inferior. O granuloma piogênico foi confirmado pelo exame histopatológico. Após o 7º e 15º dias, a região do lábio onde a biópsia foi realizada exibiu cicatrização deficiente. O caso foi discutido com o médico e foi realizada a suspensão temporária do metotrexato que resultou em cicatrização completa. A cicatrização da ferida após a suspensão temporária do metotrexato permitiu concluir que o medicamento retardou a cicatrização da ferida cirúrgica como consequência da citotoxicidade da droga. (AU)
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Humanos , Femenino , Niño , Artritis Juvenil , Metotrexato , Granuloma Piogénico , LabioRESUMEN
Abstract Introduction: Ketamine has been used as a pain management strategy, particularly in adults. There are some clinical data about the use of Ketamine in children but there are no reports referring to its efficacy, specifically in juvenile idiopathic arthritis. Ketamine could therefore become an alternate option in the management of refractory cases. Clinical findings: This report discusses the case of a 7-year-old male patient with a history of juvenile idiopathic arthritis who was admitted to the hospital as a result of an inflammatory pain crisis associated with stiff hands and feet, pain, edema, and enthesitis, in addition to signs of bilateral sacroiliitis, stiffness impairing gait, passive and active movements, poor response to multimodal analgesia with nonsteroidal antiinflammatory drugs, steroids and weak opioids. Adequate pain control and significant improvement of the child's function was achieved after initiating ketamine infusion at analgesic doses. Conclusions: This case provides valuable information about the usefulness of ketamine as a modulator of central sensitization and inflammation that could be extrapolated to a similar population of rheumatology patients.
Resumen Introducción: La ketamina se ha empleado como estrategia analgésica sobre todo en adultos. En los niños existen algunos datos clínicos sobre su uso, sin embargo, no hay reportes que hagan referencia de su efectividad en el caso específico de dolor por artritis idiopática juvenil, dejando esta como una posible alternativa de manejo en casos refractarios. Hallazgos clínicos: El presente reporte describe, el caso de un paciente masculino de 7 años con antecedente de artritis idiopática juvenil, hospitalizado por una crisis dolorosa de tipo inflamatorio asociado a rigidez en pies y manos, con dolor, edema y entesitis y signos de sacroileítis bilateral, dificultad para la marcha y rigidez que lo limitaba para movimientos activos y pasivos, poca respuesta a la analgesia multimodal con AINES, esteroides y opioides débiles, en quien se obtuvo un adecuado control del dolor y mejoría significativa de su capacidad funcional luego de iniciar infusión de ketamina en dosis analgésicas. Conclusión: Este caso nos brinda información valiosa sobre la utilidad de la ketamina como modulador de la sensibilización central e inflamación que podría ser extrapolada a una población similar de pacientes reumatológicos.
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HumanosRESUMEN
Objective In 2016,European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric rheumatology international trials organization (PRINTO) released the classification criteria for macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA).Due to the similarities of both clinical manifestations and pathogenesis between adult-onset Still dsease (AOSD) and sJIA,we hope to evaluate the 2016 sJIA-AMS classification in AOSD patients.Methods A total of 169 AOSD patients who were hospitalized in Renji Hospital were enrolled in this study.AOSD patients were divided into AOSD with MAS and AOSD without MAS,using the 2016 sJIA-MAS criteria.The data of the two groups were analyzed by Chi-square test,Mann-Whitney U test and binary Logistic analysis,and factors influencing the prognosis of patients were analyzed by Kaplan-Meier and COX regression analysis.Results According to sJIA-MAS criteria,56 AOSD patients with MAS were identified in all the 169 AOSD cases.In AOSD patients,the incidence of splenomegaly and pericarditis/myocarditis was significantly higher in patients with MAS than in AOSD without MAS [42.9％ vs 14.2％,OR(95％CI)=4.50(2.13,9.51),P＜0.01;10.7％ vs 0.9％,OR(95％CI)=13.21 (1.56,113.57),P＜0.01],also the incidence of liver dysfunction was higher in AOSD with MAS [67.8％ vs 11.5％,OR(95％CI)=0.18(7.26,36.33),P＜0.01].Among the AOSD with MAS,62.5％(35/56) of these patients received large-dose glucocorticoid therapy,5.4％ (3/56) received the glucocorticoid pulse therapy,48.2％(27/56) were treated with IVIG,and 26.8％(15/56) were treated with calcium phosphatase inhibitors.The mortality rates of AOSD with MAS was 8.9％(5/56),which was significantly higher than 1.8％(2/113) (OR =5.44,P＜0.05),the mortality rate of the AOSD without MAS.Patients who fulfilled the sJIA-MAS criteria suggested poor prognosis (OR=0.041,P=5.44),and the platelet count ≤ 181× 109/L (OR=12.17,P=0.002),alanine aminotransferase ＞48 U/L (OR=9.43,P=9.040) were also highly suggestive of poor prognosis.Conclusion The 2016 sJIA-MAS classification criteria are particularly valuable for early recognization of MAS in AOSD patients,and convenient to use.AOSD patients fulfilled sJIA-MAS criteria are more severe,and require larger doses of glucocorticoid and more immunosuppression therapy compared to patients without MAS,and the prognosis of these patients is also poor.
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Objective To compare the positive rate of anti-mutant citrulline vimentin (MCV) antibody and anti-cyclic citrullinated peptide (CCP) antibody in serum of patients with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA).To investigate the diagnostic value and significance of anti-MCV and antiCCP antibody in these two diseases.Methods Anti-CCP and anti-MCV antibodies were detected by enzymelinked immunosorbent assay (ELISA).The serum samples were from 113 patients with JIA,632 patients with RA,102 adult without RA and 56 children without RA.Chi-square test and multiple comparisons were used for statistical analysis.Results ① In RA patients,the sensitivity,specificity and area under the receiver operating characteristic curve (ROC curve) of anti-MCV antibody was 90.2％,91.2％,0.919;the sensitivity,specificity and area under the ROC curve of anti-CCP antibody was 92.6％,93.1％ and 0.934.In JIA,the specificity of antibodies was 98.2％,the sensitivity was low.Area under the ROC curve of anti-MCV antibody was 0.579.Area under the ROC curve of anti-CCP antibody was 0.561.② The positive rate of anti-MCV antibody in RA was 90.2％,which was higher than that of JIA (16.8％) (P＜0.01).The positive rate of anti-CCP antibody in RA was 92.2％,which was higher than that of JIA (14.2％) (P＜0.01).The positive rates of antiMCV antibody in JIA with RF-negative polyarthrosis,RF-positive polyarthrosis,systemic type,oligo-joint type,attachment points,unclassified was 11.8％,69.2％,14.3％,17.4％,3.6％,0.The positive rate of anti-CCP was 11.8％,61.5％,14.3％,13.0％,0 and 0 prespectively.For anti-MCV antibody,the chi-square values in patients with RA between RF-negative polyarthrosis,RF-positive olyarthrosis,systemic type,oligo-joint type,attachment points,unclassified arthritis were 160.2,4.02,34.4,102.0,165.1 and 57.0 respectively.There were significant differences between RA and all types of JIA (P＜0.05).The positive rate of anti-CCP antibody in patients with RA between RF-negative polyarthrosis,RF-positive polyarthrosis,systemic type,iligo-joint type,attachment points,unclassified arthritis were 192.3,11.9,44.0,139.4,212.5 and 71.9.There were significant differences between RA and all types of JIA (P＜0.05).Conclusion The diagnostic value of anti-MCV and anti-CCP antibodies is high in RA.Anti-MCV and anti-CCP antibody have certain diagnostic value of JIA.The positive rates of anti-MCV and anti-CCP antibody in the types in JIA are lower than those of RA patients.
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ABSTRACT Juvenile idiopathic arthritis is a term used to include all chronic childhood arthritis of unknown etiology. It is characterized by chronic inflammation persisting for at least 6 weeks, beginning before 16 years of age. The characteristics present are chronic synovitis, arthralgia, impaired joint mobility in at least one joint, and erosion with destruction of cartilage and subchondral bone, that could be associated or not with systemic involvement, according to each subtype of the disease. During the pathologic process, the temporomandibular joint can be involved by the juvenile idiopathic arthritis, resulting in severe mandibular dysfunction, with higher frequency in female patients. Initially, these lesions can show minor alterations like flattening of the condyle, erosions, and evolve to severe lesions, like destruction of the head of the condyle. We report a case of male patient who had destruction of both condyles, as a result from juvenile idiopathic arthritis. Proposed mechanisms to explain the juvenile idiopathic arthritis was reviewed. In this report the patient did not have pain or inflammatory process, and the temporomandibular diseases was the only manifestation.
RESUMO Artrite idiopática juvenil é um termo usado para incluir toda artrite infantil crônica de etiologia desconhecida. É caracterizada por uma inflamação crônica, que persiste por pelo menos 6 semanas, com início antes dos 16 anos de idade. As características presentes são sinovite crônica, artralgia, mobilidade articular diminuída em pelo menos uma articulação, e erosão com destruição da cartilagem e do osso subcondral, podendo ser associada ou não com o envolvimento sistêmico, de acordo com cada subtipo da doença. Durante o processo patológico, a articulação temporomandibular pode ser envolvida pela artrite idiopática juvenil, resultando em disfunção mandibular severa, com maior frequência em pacientes do sexo feminino. Inicialmente, estas lesões podem mostrar pequenas alterações, como achatamento do côndilo e erosões, e evoluir para lesões graves, como a destruição da cabeça do côndilo. Relatou-se o caso de um paciente do sexo masculino, que apresentou destruição de ambos os côndilos, como resultado da artrite idiopática juvenil. Os mecanismos para explicar a artrite idiopática juvenil foram revisados na literatura. Neste relato de caso, o paciente não apresentou dor e nem processo inflamatório, sendo o comprometimento da articulação temporomandibular a única manifestação.
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Humanos , Masculino , Niño , Artritis Juvenil/complicaciones , Trastornos de la Articulación Temporomandibular/etiología , Trastornos de la Articulación Temporomandibular/diagnóstico por imagen , Artritis Juvenil/patología , Radiografía Panorámica , Trastornos de la Articulación Temporomandibular/patología , Tomografía Computarizada por Rayos XRESUMEN
La artritis idiopática juvenil es una enfermedad reumática que en su forma sistémica puede afectar distintos órganos y sistemas de órganos del cuerpo humano. Las manifestaciones dermatológicas son de las más señaladas dentro de la amplia gama de manifestaciones extra articulares que pueden acompañar a la enfermedad. Se ha descrito, con bastante frecuencia, como la utilización de distintos compuestos químicos pueden ser capaces de actuar sobre el sistema inmune de los pacientes y predisponer la aparición de manifestaciones dermatológicas, e incluso, desencadenar o activar distintas enfermedades reumáticas. Se presenta el caso de un paciente de 14 años de edad con diagnóstico de artritis idiopática juvenil, el cual comienza a presentar manifestaciones dermatológicas 3 semanas después de la realización de un tatuaje
Juvenile idiopathic arthritis is a rheumatic disease that in its systemic form can affect different organs and systems of organs of the human body. Dermatological manifestations are among the most marked within the wide range of extra articular manifestations that may accompany the disease. It has been described, quite frequently, how the use of different chemical compounds may be able to act on the immune system of patients and predispose the appearance of dermatological manifestations, and even trigger or activate different rheumatic diseases. The case of a 14-year-old patient with a diagnosis of juvenile idiopathic arthritis, who begins to present dermatological manifestations 3 weeks after the completion of a tattoo, is presented
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Objective@#To evaluate the efficacy and side effects of tocilizumab for the treatment of systemic juvenile idiopathic arthritis.@*Method@#In this prospective self case-control study, the children diagnosed with refractory systemic juvenile idiopathic arthritis admitted to Department of Rheumatism and Immunology of Children's Hospital Affiliated to Capital Institute of Pediatrics from December 2013 to June 2016 were enrolled and information before and after treatment of tocilizumab was analyzed. The tocilizumab was introvenously guttae in a dose of 8-12 mg/kg every 2 weeks. Complete blood count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) were tested before and after the application of tocilizumab. Detailed clinical manifestations were recorded. All results were analyzed by χ2 test and t test.@*Result@#Forty patients with a median age of (6.6±3.7) years were enrolled, including 15 males and 25 females. All of the patients presented with fever and 38 patients got normal temperature 24-48 hours after treatment with tocilizumab. Symptoms disappeared in 13 and improved in 4 patients after treatment among the 17 patients who presented with arthritis. Within the 10 patients who manifested with rashes, 9 patients' rashes disappeared without relapse accompanied by the normalization of temperature after the treatment of tocilizumab. One patient got normal temperature but intermittently emerged rashes after symptoms of arthritis improved. In the 40 patients, 38 well tolerated tocilizumab while 2 showed rashes and chill which disappeared shortly after antianaphylaxis treatment. No severe treatment-related infection was found in any patients. According to the study, the white blood cell counts(×109/L), CRP(mg/L) and ESR(mm/1h) tested 2 weeks after the treatment with tocilizumab were significantly lower than that before treatment(12.1±1.2 vs. 16.5±1.8, 47±8 vs. 67±9, 21±5 vs. 57±6, t=2.75, 3.98, 5.22, P=0.009, 0, 0, respectively). No significant changes were found in concentration of IL-6 and TNF-α (65(207) vs. 45(137) ng/L, and 14(6) vs. 17(19)ng/L, Z=-1.247 and-1.285, P=0.212 and 0.199 respectively).@*Conclusion@#Tocilizumab is a treatment with good efficacy and safety for refractory systemic juvenile idiopathic arthritis. Adverse effects would be found in some patients.
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Objective To investigate the efficacy and safety of tocilizumab inpatients with refractory systemic'onset juvenile idiopathic arthritis (SoJIA),and to provide a new option for the treatment of this severe disease.Methods We retrospectively studied 25 cases of hospitalized patients with refractory SoJIA treated withtocilizumab,of whom 22 had data that fit for analysis,from May 2005 to February 2016.Data of 22 cases were collected retrospectively from physicians in charge of the patients.Children with SoJIA were treated with nonsteroidal antiinflammatory drugs (NSAIDs),Glucocorticoid (GC),methotrexate,cyclosporin A,etanerceptetc before,but still in high disease activity due to inadequate response were involved.Weretrospective analyzedthe laboratory test results like C'reactive protein (CRP),Erythrocyte sedimentation rate (ESR),Ferritin and other inflammatory index.Improvement of pain,fever,rash,hepatosplenomegaly and lymphadenectasis of active SoJIA (disease course ≥6 months,and inadequate response to NSAIDs and GC) after tocilizumab treatment (Body weight ≥30 kg,8 mg/kg;Body weight＜30 kg,12 mg/kg,per 4 weeks) were analyzed.Safety data of 22 cases were collected throughout the treatment period including neutropenia,infections,anaphylaxis and elevated liver enzymes etc.We also retrospectively analyzedthe dose change of GC and the long'term effect.Dichtomous paramenters were compared teween groups using thex2 test.Continuous parameters were compared using the analysis of uariance.Results In comparison to the indices before the treatment,the level of CRP [(8.7±2.2) mg/L vs (111.6±74.4) mg/L,F=5.192,P=0.002],ESR [(6.4±6.3) mm/1 h) vs (65.6±24.3) mm/1 h,F=50.393,P=0.000],white blood cell (WBC) [(8.4±2.5)×109/L vs (17.6±8.6)×109/L,F=9.321,P=0.000],Neutrophil count [(4.9±2.4)×109/L vs.(14.4±8.7)×109/L,F=10.541,P=0.000],blood platelet (PLT) [(269.5±79.2)×109/L vs (405.4± 145.3)×109/L,F=5.704,P=0.000] and globulin [(19.2±4.1) g/L vs (30.1±3.8) g/L,F=22.896,P=0.000] decreased rapidly and hemoglobin [(118.3±9.0) g/L vs (108.5±9.8) g/L,F=4.693,P=0.002] increased significantly at 24 weeks after Tocilizumab (TCZ) treatment.Clinical manifestationssuch as fever,rash,hepatosplenomegaly,joint swelling and pain were significantly improved.GC dose [(1.25±3.8) mg·kg-1·d-1 vs (16.2±12.8) mg·kg-1·d-1,F=8.21,P=0.000] were significantly reduced after TCZ treatment (P＜0.05);American College of Rheumatology (ACR) Pedi 30/50/70/90 was improved after TCZ treatment.Adverse events occurred in 3 cases of 25 children,who were not included in the statistical analysis group.Conclusion This retrospective case series has demonstrated the efficacy of tocilizumab in SoJIA,low incidence of adverse reactions.Further studies are needed to be developed because this case series haslimited sample size.
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Objective To explore the expression of inflammasomes (NLRP3,NLRP12) and related signal proteins in the peripheral blood mononuclear cells (PBMCs) of patients with juvenile idiopathic arthritis (JIA).Methods Samples of children with definite diagnosis of active JIA in Guangzhou Women and Childrens' Medical Center were collected retrospectively.Fifty-five cases were included,among whom 30 were systemic type and 25 were joint type.Blood samples of 22 healthy controls were collected at the same time.Peripheral blood single nuclear cell (PBMCs) were separated and DNA were extracted and reverse transcription (RT) to cDNA.Fluorescent quantitative polymerase chain reaction (PCR) was used to detect NLRP3,NLRP12,ASC,and capase-1 in groups and the difference in their expression between groups were analyzed.Enzyme linked immunosorbent assay (ELISA) was utilized to test plasma levels of interleukin (IL)-6 IL-1,IL-4,IL-10,and their correlation were analyzed.Results The expression of NLRP3,NLRP12,ASC,Capase-1 in the case group (general-group and joint-group) were higher than those in the control group (P＜0.05),but there was no significant difference in the expression levels between groups (P＞0.05).The IL-1 concentration of the case group (body-type group,joint-group) was higher than the control group (P=0.001,U=l) (P=0.001,U=14),however,the level of IL-4 of the case group (body-type group,joint-group) was not significantly different from the control group (U=662,P=0.13) (U=823,P=0.535),IL-I0 of the systemic group was higher than that of the control group (U=750,P=0.023),while there was no difference between groups (U=672,P=0.212).There were no significant difference in the levels of IL-1 (U=658,P=0.408),IL-4 (U=475,P=0.068),IL-10 (U=475,P=0.195) between groups.The NLRP3 mRNA relative expression levels of the case group and the ASC (r=0.44,P=0.013 4) was significant,in addition,IL-1 (P=0.001,R=0.58),erythrocyte sedimentation rate (ESR) (r=0.415,P=0.039),C reactive protein (CRP) (r=0.438,P=0.046) were positively correlated with NLRP12 relative mRNA expression level and ASC (r=0.583 7,P=0.007),CRP (r=0.46,P=0.031 6),ESR (r=0.003,P=0.56),CD8+ T (r=0.414,P=0.036).Conclusion The abnormal expression of JIA inflammasomes in peripheral blood mononuclear cells (NLRP3,NLRP12) may be associated with juvenile idiopathic arthritis.
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La fiebre de origen desconocido es una entidad infrecuente que se define como la fiebre que persiste por más de tres semanas luego de investigación clínicaintensa, con diversidad y complejidad en cuanto a su etiología, abordaje clínico yenfoque terapéutico. Innumerables casos se resuelven lastimosamente sindeterminar un diagnóstico definitivo, orientados simplemente por sintomatología y hallazgos paraclínicos incompletos. Por este motivo, en este artículo se presentael caso clínico de un paciente de 13 años de edad con fiebre intermitente de unmes de evolución a quien se le realizan estudios pertinentes durante cincosemanas de hospitalización para establecer la causa, asignándose el diagnosticode Artritis Juvenil o Enfermedad de Still por determinaciones aisladas comoalgunos síntomas referidos y el valor de ferritina encontrado sugestivo de lapatología. A partir de ello, el objetivo de este artículo es reconocer la dificultad queenmascara el abordaje diagnóstico y terapéutico de la enfermedad, especialmenteen la edad pediátrica...
Fever of unknown origin is a rare entity which is defined as fever that persists formore than three weeks of intense clinical research with diversity and complexity inits etiology, clinical approach and therapeutic approach. Countless cases areresolved without determining unfortunately a definitive diagnosis, symptoms andgeared just for paraclinical findings incomplete. For this reason, this paperpresents a case of a child under 13 years of age with intermittent fever of 39-40°Cfor one month duration who is relevant studies conducted during five weeks ofhospitalization to determine the cause, assigning the diagnosis of Stills disease bysingle determinations as some symptoms reported and ferritin value suggestive ofpathology found. From this, the aim of this paper is to recognize the difficultymasking the diagnosis and therapeutic management of the disease, especially inchildren...
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Humanos , Fiebre de Origen Desconocido , ReumatologíaRESUMEN
Juvenile idiopathic arthritis is a common connective tissue disease of children.The rate of disability is high.Therefore,early diagnosis is important.There are some study on the value of antibodies in juvenile idiopathic arthritts,such as rheumatoid factor,antiperinuclear factor,antikeratin antibody,antibodies to cyclic citrullinated peptides and so on.This review introduces the progress of them.